Spontaneous pneumomediastinum (SPM), while rare, is probably underestimated in children. Treatment targets on the underlying disease and trigger factors. The study aimed in analysis different etiology in different age groups.

Total 37 children with SPM were analyzed from two medical centers in middle Taiwan from 1994 to 2007.

Incidence of SPM in children was 1:11,726 patients at Department of Pediatric Emergency in middle Taiwan. Bimodal peak in incidence occurred in those under 7 and in those aged 15–18 years old. The Characteristic symptoms were dyspnea (64.9%), followed by chest pain (62.2%) and neck pain (40.5%); common specific physical signs were subcutaneous emphysema (SCE) (67.6%) and Hammer's sign (13.5%). Trigger factors were infection (43.2%), asthma (21.0%), esophageal rupture (5.4%), foreign body aspiration (2.7%), and diabetic ketoacidosis (2.7%). Idiopathic SPM accounted for 35.1% of patients with mean age 14.1 years. In age distribution, preschoolers (<7 years old) got SPM mostly due to lower respiratory tract infection. In adolescents, the most common etiologies were asthma and upper respiratory tract infection. Mean hospitalization was 6.4 days. Although 17 (46.0%) patients needed intensive care, nearly all had complete resolution in chest radiography before discharge.

Clinician should keep alert to incidence of SPM from these symptoms. Etiologies varied with age and treatment must target on factors and underlying disease. Pediatr. Pulmonol. 2010; 45:869–873. © 2010 Wiley-Liss, Inc.

Health-related quality of life (HRQOL) measurements provide valuable information about the psychological and social impact of treatment on patients with cystic fibrosis (CF). This study evaluated the HRQOL of Brazilian patients with CF and assessed the changes in HRQOL domains over 1 year after dornase alfa (Pulmozyme) introduction.

One hundred fifty-six stable patients with CF and 89 caregivers answered the Portuguese-validated version of the Cystic Fibrosis Questionnaire-Revised (CFQ-R) at baseline (T₀), and at 3 (T₁), 6 (T₂), 9 (T₃), and 12 (T₄) months of follow-up. Eighteen patients were excluded because they did not fulfill the inclusion criteria. The patients were analyzed in two groups: those aged 6–11 years and those aged 14 years and older. ANOVA for observed repeated results and the last observation carried forward (LOCF) method for missing data were used for the statistical analysis.

After 1 year of follow-up, there was significant improvement in respiratory symptoms (T₄ − T₀ = 8.1; 95% confidence interval (95% CI) = [2.1;14.0]; effect size (ES) = 0.35; P < 0.001), Emotional Functioning (T₄ − T₀ = 5.6; 95% CI = [1.1;10.1]; ES = 0.31; P < 0.05), Social Functioning (T₄ − T₀ = 6.0; 95% CI = [1.3;11.7]; ES = 0.31; P < 0.05), Body Image (T₄ − T₀ = 11.9; 95% CI = [4.1;19.7]; ES = 0.42; P < 0.05), and Treatment Burden (T₄ − T₀ = 5.3; 95% CI = [0.3;10.3]; ES = 0.24; P < 0.05) domains in the younger group. A significant improvement in Role Functioning (T₄ − T₀ = 6.1; 95% CI = [1.1;11.1]; ES = 0.40; P < 0.05), Body Image (T₄ − T₀ = 12.6; 95% CI = [3.5;21.7]; ES = 0.46; P < 0.05), and Weight (T₄ − T₀ = 11.7; 95% CI = [1.8;21.6]; ES = 0.40; P < 0.05) was obtained in the older group. The caregivers' CFQ-R showed improvements in the Digestive Symptoms (T₄ − T₀ = 5.5; 95% CI = [1.5;9.4]; ES = 0.30; P < 0.05), Respiratory Symptoms (T₄ − T₀ = 7.6; 95% CI = [3.9;11.4]; ES = 0.48; P < 0.05), and Weight (T₄ − T₀ = 10.1; 95% CI = [1.6;18.6]; ES = 0.26; P < 0.05) domains.

The introduction of dornase alfa improved the HRQL of the patients with CF during the first year of treatment. Pediatr. Pulmonol. 2010; 45:874–882. © 2010 Wiley-Liss, Inc.

Invasive community acquired (CA) Staphylococcus aureus (SA) disease has been endemically observed in Hawaiian children. We wanted to evaluate the clinical, laboratory findings, and outcomes of methicillin-resistant SA (MRSA) and methicillin-susceptible SA (MSSA) associated pneumonia admissions.

We performed retrospective chart reviews of 38 culture proven SA pneumonia patients admitted to a pediatric tertiary medical center in Hawaii between January 1996 to December 2007.

Twenty-six patients (68%) had MRSA and 12 patients (32%) had MSSA infection. The mean age of MRSA patients was 2.8 and 6.7 years for MSSA patients (P < 0.05). Pacific Islander and Native Hawaiian patients were affected disproportionately compared to non-Pacific Islander and Hawaiian groups (P < 0.0001). Demographic data, days of fever, tachypnea, hypoxia, and length of stay (LOS) were not significantly different between MRSA and MSSA infected patients. The mean LOS was 26.2 days (range 6–138 days); mean length of fever was 12.4 days. Seventy five percent (15 of 20) of patients who required intubation had MRSA. Twenty-one of the 29 (72%) total patients with pleural effusions had MRSA infection and all required chest tube placements. Two (5%) patients died; both had MRSA infection.

Younger Pacific Islander/Native Hawaiian children were affected disproportionately and had MRSA infection more frequently. MRSA infected patients appeared to have severe disease with frequent chest tube placement, intubation, and fatality. Overall, both MRSA and MSSA pneumonia resulted in prolonged hospitalization, multiple complications, and significant healthcare costs. Pediatr. Pulmonol. 2010; 45:898–905. © 2010 Wiley-Liss, Inc.

Reliable interpretation of pulmonary function tests relies on appropriate reference data, which remain very limited for infants.

This study aimed to assess the validity of published reference equations for forced expiratory flow-volume (FEFV) data in infants when using current, commercially available equipment, and how this could impact on interpretation of results from infants with lung disease.

The Jaeger Masterscreen BabyBody (v4.67) equipment was used to perform partial and raised volume FEFV maneuvers in healthy infants and those with cystic fibrosis (CF). Results were initially expressed as Z-scores using published reference equations. Multilevel modeling was used to calculate differences, if any, from predicted scores in healthy infants.

Data were available from 66 healthy full term infants on 89 test occasions; [median (range) postnatal age 49.4 (12–101) weeks. All FEFV outcomes were significantly lower than predicted, with mean (SD) Z-score differences of −0.4 (1.1) for FVC; −0.6 (1.0) for FEV_0.5; −1.0 (1.0) for FEF_25–75 and −1.4 (1.1) for V'_maxFRC. After adjustments using multilevel modeling, mean Z-scores were within 0.1 (SD∼1.0) predicted for all outcomes in healthy infants. Among 50 infants with CF, studied on 85 test occasions, results were “abnormal” (<−1.96 Z-scores) on 35 (41%) and 37 (45%) test occasions for FEV_0.5 and FEF_25–75, respectively, when using published equations. This fell to 24 (28%) and 20 (24%), respectively, after adjustment.

Dependence on published equations for interpreting FEFV data in infants may lead to misinterpretation of lung function status, which could impact adversely both in the research setting and on clinical management. Use of a contemporary control group or establishment of equipment-specific reference data is essential for meaningful interpretation of infant lung function data. Pediatr. Pulmonol. 2010; 45:906–913. © 2010 Wiley-Liss, Inc.

There is very limited information on how the risk of persistent asthma in recurrent wheezing (RW) infants modifies their lung function early in life. The aim of this study is to compare lung function of RW infants and young children with a positive or negative asthma predictive index (API), an index previously used to anticipate asthma persistence into childhood and adolescence.

Two groups of RW infants and young children were recruited in two centres in Spain (Palma de Mallorca and Murcia). Lung function was measured according to the thoracho-abdominal compression technique (RCT), and values of the maximal flow at functional residual capacity were expressed as Z-scores. Other variables included in the study, as independent factors, were: gender, age, length, weight, and parental smoking habits together with information regarding API.

Expressed as mean ± SD, API+ RW infants (n = 50; age in months 11.9 ± 4.9) had a lower Z-score than API− RW ones (n = 41; age in months 12.3 ± 6.2; −2.01 ± 0.79 vs. −1.64 ± 0.77, P = 0.026, respectively). Centre and tobacco exposition did not have an effect on lung function.

Among RW infants and young children, those having a positive API have a significant lower lung function as measured by at an early age. Pediatr. Pulmonol. 2010; 45:914–918. © 2010 Wiley-Liss, Inc.

Asthma therapy should be stepped up or stepped down in response to changes in asthma control. However, there is little evidence available on the optimal timing, sequence, and degree of medication reductions. In this study we analyzed clinically stable asthmatic children who underwent a medication reduction from a combination preparation consisting of an inhaled corticosteroid (ICS) and long acting beta2-agonist (LABA) to monotherapy with the same dose of the ICS. We hypothesized that the extent of exercise-induced bronchoconstriction (EIB) would not increase after the cessation of the LABA.

Nineteen children, aged 8–16 years, with clinically stable asthma, receiving LABA/ICS combination therapy, were analyzed in this open-label pilot study. Children performed an exercise challenge at baseline and 3 weeks after the medication reduction. Best values of spirometric measurements of the forced expiratory volume in 1 sec (FEV₁) were used for statistical calculations.

Maximum percent fall in FEV₁ was significantly lower after 3 weeks of ICS monotherapy (P = 0.03). Eight of 19 children had a ≥15% fall in FEV₁ after exercise at the initial exercise challenge. In this subgroup, maximum percent fall in FEV₁ after the medication reduction was significantly lower (P < 0.01), and in six children it decreased to <15%, indicating they no longer had EIB.

In clinically stable asthmatic children on LABA/ICS combination therapy, the cessation of the LABA can reduce and in most cases abolish EIB. Pediatr. Pulmonol. 2010; 45:927–933. © 2010 Wiley-Liss, Inc.

The EPIC Observational Study is an ongoing prospective cohort study investigating risk factors for and clinical outcomes associated with early Pseudomonas aeruginosa (Pa) acquisition in young children with cystic fibrosis (CF).

To describe the baseline characteristics of the cohort and evaluate associations between potential risk factors and nutritional and respiratory characteristics at enrollment. We hypothesized that distinct demographic and environmental risk factors could be identified for poorer nutritional status and lung function at enrollment.

During 2004–2006, 1,700 children with CF were enrolled at 59 US CF centers. Children ≤12 years were eligible if they had no prior Pa infection (Pa-Never) or, if prior isolation of Pa from respiratory cultures, at least a 2-year history of Pa negative cultures (Pa-Past).

One thousand one hundred seventeen participants (65.7%) were Pa-Never and 583 (34.3%) Pa-Past. Pa-never patients had a lower proportion of CFTR genotypes with both mutations in functional classes I, II, or III), higher lung function and less respiratory symptoms. Diagnosis after newborn or prenatal screening was associated with significantly higher mean weight, height, and FEV₁ at enrollment, while maternal smoking during pregnancy appeared to worsen these parameters.

Children in this cohort with a remote history of Pa infection had a higher proportion of CFTR genotypes associated with severely reduced CFTR function as well as lower lung function and more respiratory symptoms than those without prior Pa infection. These observed differences in respiratory indices may reflect the impact of prior Pa airway infection and/or of CFTR genotype or other genetic factors predisposing both to earlier Pa acquisition and more severe lung disease. Key characteristics associated with nutritional and pulmonary status at enrollment included diagnosis after prenatal or neonatal screening (protective) and in utero cigarette exposure (harmful). Pediatr. Pulmonol. 2010; 45:934–944. © 2010 Wiley-Liss, Inc.